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Related articles

Clinical manifestations and diagnosis of pulmonary sarcoidosis

INTRODUCTION

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that affects individuals worldwide and is characterized pathologically by the presence of noncaseating granulomas in involved organs. It typically affects young adults, and initially presents with one or more of the following abnormalities:

Bilateral hilar adenopathy

Pulmonary reticular opacities

Skin, joint, and/or eye lesions

An overview of the clinical manifestations and diagnosis of pulmonary sarcoidosis is presented here. Issues relating to extrapulmonary sarcoidosis and the pathogenesis and treatment of sarcoidosis are discussed separately. (See "Pathogenesis of sarcoidosis" and "Treatment of pulmonary sarcoidosis with glucocorticoids" and "Treatment of pulmonary sarcoidosis with alternatives to glucocorticoids" and "Extrapulmonary manifestations of sarcoidosis".)

                            

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Literature review current through: Oct 2014. | This topic last updated: Feb 28, 2014.
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References
Top
  1. Thomas KW, Hunninghake GW. Sarcoidosis. JAMA 2003; 289:3300.
  2. Rybicki BA, Major M, Popovich J Jr, et al. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 1997; 145:234.
  3. Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet 2003; 361:1111.
  4. Martinetti M, Tinelli C, Kolek V, et al. "The sarcoidosis map": a joint survey of clinical and immunogenetic findings in two European countries. Am J Respir Crit Care Med 1995; 152:557.
  5. Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997; 336:1224.
  6. Morimoto T, Azuma A, Abe S, et al. Epidemiology of sarcoidosis in Japan. Eur Respir J 2008; 31:372.
  7. Johnson LA, Edsall JR, Austin JH, Ellis K. Pulmonary sarcoidosis: could mycoplasma-like organisms be a cause? Sarcoidosis Vasc Diffuse Lung Dis 1996; 13:38.
  8. Bocart D, Lecossier D, De Lassence A, et al. A search for mycobacterial DNA in granulomatous tissues from patients with sarcoidosis using the polymerase chain reaction. Am Rev Respir Dis 1992; 145:1142.
  9. Rybicki BA, Iannuzzi MC, Frederick MM, et al. Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS). Am J Respir Crit Care Med 2001; 164:2085.
  10. Judson MA, Boan AD, Lackland DT. The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis Vasc Diffuse Lung Dis 2012; 29:119.
  11. Baughman RP, Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164:1885.
  12. Rizzato G, Tinelli C. Unusual presentation of sarcoidosis. Respiration 2005; 72:3.
  13. Rizzato G, Palmieri G, Agrati AM, Zanussi C. The organ-specific extrapulmonary presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis Vasc Diffuse Lung Dis 2004; 21:119.
  14. Sharma OP. Fatigue and sarcoidosis. Eur Respir J 1999; 13:713.
  15. Chevalet P, Clément R, Rodat O, et al. Sarcoidosis diagnosed in elderly subjects: retrospective study of 30 cases. Chest 2004; 126:1423.
  16. Spruit MA, Thomeer MJ, Gosselink R, et al. Skeletal muscle weakness in patients with sarcoidosis and its relationship with exercise intolerance and reduced health status. Thorax 2005; 60:32.
  17. Delobbe A, Perrault H, Maitre J, et al. Impaired exercise response in sarcoid patients with normal pulmonary functio. Sarcoidosis Vasc Diffuse Lung Dis 2002; 19:148.
  18. Miller A, Brown LK, Sloane MF, et al. Cardiorespiratory responses to incremental exercise in sarcoidosis patients with normal spirometry. Chest 1995; 107:323.
  19. Pattishall EN, Kendig EL Jr. Sarcoidosis in children. Pediatr Pulmonol 1996; 22:195.
  20. Milman N, Hoffmann AL. Childhood sarcoidosis: long-term follow-up. Eur Respir J 2008; 31:592.
  21. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999; 160:736.
  22. Judson MA. The diagnosis of sarcoidosis. Clin Chest Med 2008; 29:415.
  23. de Prost N, Kerrou K, Sibony M, et al. Fluorine-18 fluorodeoxyglucose with positron emission tomography revealed bone marrow involvement in sarcoidosis patients with anaemia. Respiration 2010; 79:25.
  24. Lower EE, Smith JT, Martelo OJ, Baughman RP. The anemia of sarcoidosis. Sarcoidosis 1988; 5:51.
  25. Yanardağ H, Pamuk GE, Karayel T, Demirci S. Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples. Haematologia (Budap) 2002; 32:419.
  26. Renston JP, Goldman ES, Hsu RM, Tomashefski JF Jr. Peripheral blood eosinophilia in association with sarcoidosis. Mayo Clin Proc 2000; 75:586.
  27. Spagnolo P, Cullinan P, duBois RM. Sarcoidosis. In: Interstitial Lung Disease, 5th, Schwarz MI, King TE Jr. (Eds), People's Medical Publishing House, Shelton, CT 2011. p.433.
  28. Marcellis RG, Lenssen AF, Elfferich MD, et al. Exercise capacity, muscle strength and fatigue in sarcoidosis. Eur Respir J 2011; 38:628.
  29. Sweiss NJ, Barnathan ES, Lo K, et al. C-reactive protein predicts response to infliximab in patients with chronic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2010; 27:49.
  30. Studdy PR, Bird R. Serum angiotensin converting enzyme in sarcoidosis--its value in present clinical practice. Ann Clin Biochem 1989; 26 ( Pt 1):13.
  31. Baughman RP. Pulmonary sarcoidosis. Clin Chest Med 2004; 25:521.
  32. Semenzato C, Facco M, Agostini C. Immunologic events in the pathogenesis of interstitial lung disease: the paradigm of sarcoidosis. In: Interstitial Lung Disease, 5th, Schwarz, MI, King, TE JR (Eds), People's Medical Publishing House, Shelton, CT 2011. p.407.
  33. Hours S, Nunes H, Kambouchner M, et al. Pulmonary cavitary sarcoidosis: clinico-radiologic characteristics and natural history of a rare form of sarcoidosis. Medicine (Baltimore) 2008; 87:142.
  34. Soskel NT, Sharma OP. Pleural involvement in sarcoidosis. Curr Opin Pulm Med 2000; 6:455.
  35. Navaneethan SD, Venkatesh S, Shrivastava R, et al. Recurrent pleural and pericardial effusions due to sarcoidosis. PLoS Med 2005; 2:e63.
  36. Huggins JT, Doelken P, Sahn SA, et al. Pleural effusions in a series of 181 outpatients with sarcoidosis. Chest 2006; 129:1599.
  37. Koyama T, Ueda H, Togashi K, et al. Radiologic manifestations of sarcoidosis in various organs. Radiographics 2004; 24:87.
  38. Malaisamy S, Dalal B, Bimenyuy C, Soubani AO. The clinical and radiologic features of nodular pulmonary sarcoidosis. Lung 2009; 187:9.
  39. Nishimura K, Itoh H, Kitaichi M, et al. Pulmonary sarcoidosis: correlation of CT and histopathologic findings. Radiology 1993; 189:105.
  40. Müller NL, Miller RR. Ground-glass attenuation, nodules, alveolitis, and sarcoid granulomas. Radiology 1993; 189:31.
  41. Teirstein AS, Machac J, Almeida O, et al. Results of 188 whole-body fluorodeoxyglucose positron emission tomography scans in 137 patients with sarcoidosis. Chest 2007; 132:1949.
  42. Kaira K, Oriuchi N, Otani Y, et al. Diagnostic usefulness of fluorine-18-alpha-methyltyrosine positron emission tomography in combination with 18F-fluorodeoxyglucose in sarcoidosis patients. Chest 2007; 131:1019.
  43. Line BR, Hunninghake GW, Keogh BA, et al. Gallium-67 scanning to stage the alveolitis of sarcoidosis: correlation with clinical studies, pulmonary function studies, and bronchoalveolar lavage. Am Rev Respir Dis 1981; 123:440.
  44. Schoenberger CI, Line BR, Keogh BA, et al. Lung inflammation in sarcoidosis: comparison of serum angiotensin-converting enzyme levels with bronchoalveolar lavage and gallium-67 scanning assessment of the T lymphocyte alveolitis. Thorax 1982; 37:19.
  45. Beaumont D, Herry JY, Sapene M, et al. Gallium-67 in the evaluation of sarcoidosis: correlations with serum angiotensin-converting enzyme and bronchoalveolar lavage. Thorax 1982; 37:11.
  46. Baughman RP, Shipley R, Eisentrout CE. Predictive value of gallium scan, angiotensin-converting enzyme level, and bronchoalveolar lavage in two-year follow-up of pulmonary sarcoidosis. Lung 1987; 165:371.
  47. Shorr AF, Helman DL, Lettieri CJ, et al. Depreotide scanning in sarcoidosis: a pilot study. Chest 2004; 126:1337.
  48. Lebtahi R, Crestani B, Belmatoug N, et al. Somatostatin receptor scintigraphy and gallium scintigraphy in patients with sarcoidosis. J Nucl Med 2001; 42:21.
  49. Dunn TL, Watters LC, Hendrix C, et al. Gas exchange at a given degree of volume restriction is different in sarcoidosis and idiopathic pulmonary fibrosis. Am J Med 1988; 85:221.
  50. Baughman RP, Sparkman BK, Lower EE. Six-minute walk test and health status assessment in sarcoidosis. Chest 2007; 132:207.
  51. Karetzky M, McDonough M. Exercise and resting pulmonary function in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 1996; 13:43.
  52. Lin YH, Haslam PL, Turner-Warwick M. Chronic pulmonary sarcoidosis: relationship between lung lavage cell counts, chest radiograph, and results of standard lung function tests. Thorax 1985; 40:501.
  53. Ward K, O'Connor C, Odlum C, Fitzgerald MX. Prognostic value of bronchoalveolar lavage in sarcoidosis: the critical influence of disease presentation. Thorax 1989; 44:6.
  54. Winterbauer RH, Lammert J, Selland M, et al. Bronchoalveolar lavage cell populations in the diagnosis of sarcoidosis. Chest 1993; 104:352.
  55. Agostini C, Trentin L, Zambello R, et al. CD8 alveolitis in sarcoidosis: incidence, phenotypic characteristics, and clinical features. Am J Med 1993; 95:466.
  56. Kantrow SP, Meyer KC, Kidd P, Raghu G. The CD4/CD8 ratio in BAL fluid is highly variable in sarcoidosis. Eur Respir J 1997; 10:2716.
  57. Perez RL, Duncan A, Hunter RL, Staton GW Jr. Elevated D dimer in the lungs and blood of patients with sarcoidosis. Chest 1993; 103:1100.
  58. Perez RL, Kimani AP, King TE Jr, et al. Bronchoalveolar lavage fluid D dimer levels are higher and more prevalent in black patients with pulmonary sarcoidosis. Respiration 2007; 74:297.
  59. Meyer KC, Raghu G, Baughman RP, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med 2012; 185:1004.
  60. Polychronopoulos VS, Prakash UB. Airway involvement in sarcoidosis. Chest 2009; 136:1371.
  61. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 Suppl 5:v1.
  62. Bjermer L, Thunell M, Rosenhall L, Stjernberg N. Endobronchial biopsy positive sarcoidosis: relation to bronchoalveolar lavage and course of disease. Respir Med 1991; 85:229.
  63. de Boer S, Milne DG, Zeng I, Wilsher ML. Does CT scanning predict the likelihood of a positive transbronchial biopsy in sarcoidosis? Thorax 2009; 64:436.
  64. Descombes E, Gardiol D, Leuenberger P. Transbronchial lung biopsy: an analysis of 530 cases with reference to the number of samples. Monaldi Arch Chest Dis 1997; 52:324.
  65. Roethe RA, Fuller PB, Byrd RB, Hafermann DR. Transbronchoscopic lung biopsy in sarcoidosis. Optimal number and sites for diagnosis. Chest 1980; 77:400.
  66. Keogh BA, Hunninghake GW, Line BR, Crystal RG. The alveolitis of pulmonary sarcoidosis. Evaluation of natural history and alveolitis-dependent changes in lung function. Am Rev Respir Dis 1983; 128:256.
  67. Takemura T, Hiraga Y, Oomichi M, et al. Ultrastructural features of alveolitis in sarcoidosis. Am J Respir Crit Care Med 1995; 152:360.
  68. Ma Y, Gal A, Koss MN. The pathology of pulmonary sarcoidosis: update. Semin Diagn Pathol 2007; 24:150.
  69. Myers JL, Tazelaar HD. Challenges in pulmonary fibrosis: 6--Problematic granulomatous lung disease. Thorax 2008; 63:78.
  70. Mukhopadhyay S, Wilcox BE, Myers JL, et al. Pulmonary necrotizing granulomas of unknown cause: clinical and pathologic analysis of 131 patients with completely resected nodules. Chest 2013; 144:813.
  71. Aubry MC. Necrotizing granulomatous inflammation: what does it mean if your special stains are negative? Mod Pathol 2012; 25 Suppl 1:S31.
  72. Brincker H. Coexistence of sarcoidosis and malignant disease: causality or coincidence? Sarcoidosis 1989; 6:31.
  73. Ojeda H, Sardi A, Totoonchie A. Sarcoidosis of the breast: implications for the general surgeon. Am Surg 2000; 66:1144.
  74. Toner GC, Bosl GJ. Sarcoidosis, "Sarcoid-like lymphadenopathy," and testicular germ cell tumors. Am J Med 1990; 89:651.
  75. Nakamura M, Iemura A, Kojiro M, et al. Leiomyosarcoma of the rectum with sarcoid-like reaction--a case report. Kurume Med J 1990; 37:171.
  76. Campbell F, Douglas-Jones AG. Sarcoid-like granulomas in primary renal cell carcinoma. Sarcoidosis 1993; 10:128.
  77. Newman TG, Minkowitz S, Hanna A, et al. Coexistent sarcoidosis and HIV infection. A comparison of bronchoalveolar and peripheral blood lymphocytes. Chest 1992; 102:1899.
  78. Haramati LB, Lee G, Singh A, et al. Newly diagnosed pulmonary sarcoidosis in HIV-infected patients. Radiology 2001; 218:242.
  79. Wildner D, Boxberger F, Wein A, et al. Granulomatous lung disease requiring mechanical ventilation induced by a single application of oxaliplatin-based chemotherapy for colorectal cancer: a case report. Case Rep Oncol Med 2013; 2013:683948.
  80. Sekhri A, Palaniswamy C, Kurmayagari K, et al. Interstitial lung disease associated with azacitidine use: a case report. Am J Ther 2012; 19:e98.
  81. Vital Durand D, Durieu I, Rousset H. [Toxic or drug-induced granulomatous reactions]. Rev Med Interne 2008; 29:33.
  82. Howard L, Gopalan D, Griffiths M, Mahadeva R. Sirolimus-induced pulmonary hypersensitivity associated with a CD4 T-cell infiltrate. Chest 2006; 129:1718.
  83. de Kerviler E, Trédaniel J, Revlon G, et al. Fluoxetin-induced pulmonary granulomatosis. Eur Respir J 1996; 9:615.
  84. Chang H, Klein JS, Norotsky M, Cooper K. Granulomatous chest disease following intravesical bacillus Calmette-Guerin immunotherapy. J Thorac Imaging 2004; 19:60.
  85. Judson MA, Baughman RP, Teirstein AS, et al. Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS Research Group. A Case Control Etiologic Study of Sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 1999; 16:75.
  86. Burke RR, Rybicki BA, Rao DS. Calcium and vitamin D in sarcoidosis: how to assess and manage. Semin Respir Crit Care Med 2010; 31:474.
  87. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007; 357:2153.